Symptoms Of Pancreatic Insufficiency Research Articles

P340 The impact of modulators on faecal elastase in children with cystic fibrosis

CFTR modulators, improve lung function, body mass index, and reduce pulmonary exacerbations. There is some evidence from clinical trials 1 Rosenfeld et al. ARRIVAL. Lancet Resp Med. 2018 Jul; 6 Google Scholar that CFTR modulators may restore some degree of pancreatic function, but this has not been thoroughly investigated in the “real world.” A number of children from our centre’s cohort, a large tertiary paediatric hospital in Northwest England, have reported a reduction in the symptoms of pancreatic insufficiency since commencing modulator therapy.

PS02.160: OUTPATIENT FOLLOW-UP IN OESOPHAGO-GASTRIC SURGERY PATIENTS: A SAFE AND COST-EFFECTIVE ALLIED HEALTHCARE PROFESSIONAL MODEL

Abstract Background Post-operative follow-up for post oesophago-gastric resection patients are usually conducted by clinicians. Follow-up normally continues for a 5-year period. In high volume tertiary NHS centres, there is an increased demand for outpatient clinician episodes. We have successfully trialled and implemented a model using Allied Healthcare Professionals (AHP); consisting of a Cancer Nurse Specialist (CNS) and Specialist Dietician (SD). We propose that AHP follow-up in the outpatient department is cost-effective and beneficial to patients. Methods An AHP clinic was implemented in our regional tertiary unit in February 2017. All post-operative patients without any on-going complications were eligible to attend the AHP clinic from their second outpatient visit. Data was collected from February 2017 to January 2018. The data was prospectively collected and retrospectively analysed. The AHP follow-up clinic was conducted by a CNS & SD, which runs in parallel with a consultant led clinic. Each patient in the AHP clinic is given an extended 30-minute consultation, compared to a standard 10-minute consultant appointment. This allows for queries to be addressed thoroughly and investigations required to be made. Results During this initial trial period, there were 44 AHP clinics with 197 outpatient consultations; average of 4.5 patients per clinic. There was good outpatient attendance compliance and improved patient satisfaction. In more recent patients, assessment for pancreatic insufficiency, iron, folate, B12 and vitamin D deficiencies were assessed and treated. 27 of 77 (35%) had symptoms of pancreatic insufficiency, 6 of 57 (12%) had low folate, 4 of 52 (7%) had B12 deficiency and 6 of 26 (23%) had vitamin D deficiency. All patients with deficiencies were given supplementation. Conclusion AHP follow-up from their second visit is safe, improves patient contact and satisfaction. Nutritional deficiencies can be detected and treatment commenced by AHPs without detriment to patient outcome. This provides a platform for cost effective use of resources. AHP follow-up increases consultant outpatient episodes available for the hospital trust. In the next phase, the AHPs intend on having 270 patient consultations per year (6 patient consultations per clinic with 45 clinics per year) with a standardised nutritional deficiency assessment. Disclosure All authors have declared no conflicts of interest.

Glycemic Control in Patients Post Total Pancreatectomy (TP) for Intraductal Papillary Mucinous Neoplasm (IPMN)

Purpose: Diabetes Mellitus (DM) after total pancreatectomy (TP) is often thought to be difficult to manage. The notion that it causes brittle diabetes in up to 25% could adversely influence the decision to perform TP which is other wise the treatment of choice for main duct or multifocal IPMN. It is still not clear if that has changed with recent medical advances, or if the underlying pancreatic disease affects glycemic control post TP. Only one French study of 10 patients found that glycemic control can be managed successfully after TP for mucinous pancreatic tumors. Methods: A retrospective chart review of patients that have undergone TP for IPMN at our institution to evaluate glycemic control Results: We identified 15 patients who underwent TP for IPMN. The mean age was 69 (range 47–78), 3 males, 12 females. Three patients were excluded because the available follow up data was less than 2 months. The mean follow was 20 months (2–43 months), mean BMI at time of surgery 24.8 Kg/m2 (19–35.5), and approximately 1 year out was 21.4 Kg/m2 (15.8–32, data on 10 patients). Only 1 patient had DM type 2 prior to surgery, and 2 patients had symptoms of pancreatic insufficiency. All patients were started on an insulin drip post surgery, and were discharged on a sliding scale. In addition, patients were given either Lantus insulin (9 patients), Insulin 70/30 (1 patient), or Levemir (1 patient) based on the discretion of the endocrinologist. Patients were also discharged on pancreatic enzyme supplements. Only one patient developed hypoglycemia that required admission to the emergency room, where she was treated with intravenous Dextrose 50% and discharged home. Other patients had occasional hypoglycemia noted during blood sugar monitoring, with minimal or no symptoms at home. All these episodes were managed by the patients, none requiring glucagon therapy or hospital admission. Current insulin regimens include Lantus insulin (7 patients, mean dose 11 U/day, range 4–24), insulin pump (3 patients), Humalin (1 patient), and levemir (1 patient). The most recent HbA1c mean was 7.28 (range 5.2–8.6, data for 10 patients), and overall mean HbA1c was 7.3 (range 5.85–8.2, data for 10 patients). Most patients continued on pancreatic enzyme supplements to avoid malabsorption, with its potential negative effects on glycemic control. Only 2 patients continued to complain of steatorrhea because of intolerance of medications (1), and inadequate dosing (1). Conclusion: Glycemic control following TP for IPMN can be well managed and controlled with a variety of insulin therapy regimens. Fear of DM following TP for IPMN should not preclude surgery when indicated.

Shwachman-Diamond Syndrome: Clinical Phenotypes

The clinical phenotype of Shwachman-Diamond syndrome (SDS) is extremely heterogeneous, showing a wide range of abnormalities and symptoms. The main characteristics of the syndrome are exocrine pancreatic dysfunction, haematologic abnormality and growth retardation. At diagnosis, especially when made in infancy, symptoms of pancreatic insufficiency are always present. This condition could be considered as a transient pancreatic insufficiency. In fact, several studies have shown that, with advancing age, about 40–60% of patients become pancreatic sufficient. Observations on the evolution of pancreatic activity lead us to believe that the diagnosis of SDS must be considered even in the absence of signs and symptoms of pancreatic insufficiency. Intermittent neutropenia is the most common haematological finding in SDS, but more of the bone marrow cellular elements can be involved. In recent years, recombinant human granulocyte colony-stimulating factor has been used in some SDS subjects with severe neutropenia and frequent infection. The major haematological problem in the disease is the appearance of acute myeloid leukaemia; however, its prevalence is difficult to establish. Growth retardation is a typical manifestation. Weight and length are deficient at birth and remain below normal over time. Some studies show that SDS patients present short stature rather than malnutrition and this would suggest an inherent growth problem. A broad spectrum of skeletal abnormalities has been found to be associated with this syndrome. Short ribs with broadened anterior ends and metaphyseal dyschondroplasia of the long bone are the most common findings. Elevated liver enzymes and hepatomegaly are present in the first years of life with subsequent improvement without complications. Developmental delay, learning disorders and attention deficit disorders are also reported.

Evaluation of pancreatic exocrine function using pure pancreatic juice in noninsulin-dependent diabetes mellitus.

To investigate the pancreatic exocrine function in noninsulin-dependent diabetes mellitus (type 2 DM), we evaluated the pure pancreatic juice obtained by endoscopic cannulation of the main pancreatic duct in 13 healthy control subjects and 22 patients with type 2 DM who had no evidence of pancreatic disease. Samples of pancreatic juices were collected in six fractions for 30 minutes at 5-minute intervals after an intravenous bolus injection of secretin (0.25 CU/kg) and cholecystokinin-8 (CCK) (40 ng/kg). The responses of plasma glucose, insulin, and C-peptide to intravenous administration of glucose (50%, 40 mL) were measured. The levels of plasma insulin and C-peptide levels in type 2 DM were the same as in healthy controls in the basal state but did not further increase in response to an intravenous glucose. This suggested that patients with type 2 DM had insulin secretion defect rather than insulin deficiency. Pancreatic secretions including volume, bicarbonate, and protein output in response to stimulation with secretin, and CCK were significantly reduced when compared to the healthy controls. We conclude that patients with type 2 DM exhibit impairment of pancreatic exocrine secretion and that this impairment might not be related to insulin deficiency. Therefore, we recommended that careful evaluation for exocrine pancreatic function in type 2 diabetics who have any clinically suspicious symptoms of pancreatic insufficiency.

Management of Patients with Cystic Fibrosis

Cystic fibrosis (CF) is a serious genetically inherited multisystem disease, characterised by symptoms of pancreatic insufficiency, meconium ileus, obstruction/absence of the vas deferens and chronic obstruction, infection and inflammation of the lower respiratory tract. Lung disease, specifically chronic infection caused by Pseudomonas aeruginosa, is the principal cause of morbidity and mortality in patients with CF.

Failure to thrive: the earliest feature of cystic fibrosis in infants diagnosed by neonatal screening.

The benefits of early treatment of nutritional and respiratory problems in the CF infant and of genetic counselling for the parents are widely recognized. However, clinical diagnosis of CF is often delayed despite early onset of symptoms and the usefulness of neonatal population screening as a preventive measure is still under debate. This study analyses the clinical history of CF patients diagnosed exclusively on the basis of positive neonatal screening tests with the aim of identifying the earliest markers of the disease. We studied 103 CF infants born in north-east Italy, diagnosed following neonatal screening: assay of immunoreactive trypsin (IRT) from a heel-prick blood sample followed by a measurement of meconium lactase in cases with raised IRT. Diagnosis was confirmed by sweat test at an average age of 39 days. Eighty-one patients (79%) had symptoms strongly suggestive of CF at diagnosis, and signs and/or symptoms of pancreatic insufficiency were present in 16 of the remaining 22 cases. The most frequent symptom was growth failure (69% of infants) and of these, 44% weighed the same as at birth or less. Pancreatic insufficiency was confirmed by the low level of faecal chymotrypsin found in 85% of cases. IRT was elevated in all cases. CF had not been suspected in any symptomatic infant, although most of the infants had been monitored by a paediatrician. In conclusion, most infants with CF diagnosed by neonatal screening are already symptomatic in the first six weeks of life and the most frequent symptom is failure to thrive; pancreatic insufficiency was already present in most cases. In areas without CF neonatal screening programs, the disease should be excluded by differential diagnosis in all cases with growth failure notwithstanding adequate caloric intake in the first months of life. The high sensitivity, low cost and simple execution of IRT and fecal chymotrypsin tests make them an ideal first step in suspect cases before proceeding to the sweat test, often performed late because of limited availability.

Association of pancreatic insufficiency and chronic neutropenia in childhood.

In childhood, pancreatic exocrine insufficiency is rarely encountered except as one of the features of cystic fibrosis, which is in contrast to the pattern of pancreatic disease in adults, where pancreatitis is common. However, paediatricians recognize that some children with symptoms of pancreatic insufficiency do not have the chronic chest infection and raised levels of electrolytes in the sweat characteristic of cystic fibrosis. Recently Bodian, Sheldon, and Lightwood (1964) have described congenital hypoplasia of the exocrine pancreas in 2 such children. Variable neutropenia and thrombocytopenic purpura occurred in both but did not receive special comment. In 1964 Shwachman, Diamond, Oski, and Khaw reported 5 similar patients, drawing particular attention to the association of haematological and pancreatic abnormalities. These reports stimulated us to review those of our patients whom we knew to have pancreatic exocrine insufficiency but not the other findings of cystic fibrosis, and those children with so-called idiopathic neutropenia. Two of us (C.M.A. and J.H.C.) had recognized as far back as 1946 one patient with both pancreatic insufficiency and chronic neutropenia, but had not pursued this association. A preliminary report of some of the findings in these patients has been published (Colebatch, Anderson, Simons, and Burke, 1965), but further patients have been encountered since that time. In all, 19 children have presented with, initially, at least one of these features, and 11 of them have now been shown to have both. Although the syndrome is rare, we consider it to be the major disorder of pancreatic exocrine function of children in whom cystic fibrosis has been excluded. Associated features are noteworthy in a number of the patients, particularly the radiological changes of metaphysial dysostosis. A familial incidence of the

Characteristics of Cystic Fibrosis in Adults A Report of Seven Patients

(1) The chief diagnostic clues observed in seven cases of adult cystic fibrosis were: A characteristic roentgen appearance associated with bronchographic signs of bronchitis; recurrent staphylococcal infection of the respiratory tract; and evidence of diffuse airway obstruction in the absence of bronchial asthma. (2) Symptoms of pancreatic insufficiency were mild or absent in four of five patients who had laboratory evidence of intestinal malabsorption. (3) Spontaneous prolonged remission of Symptoms and a relatively benign course occurred in one instance. (4) The inability of a fibrocystic patient to conserve sweat Chloride during administration of desoxycorticosterone or salt deprivation was confirmed, and provides another useful diagnostic test for disease.